가와사키병(kawasaki disease)

소개

가와사키병에 대한 disease reivew입니다.
소아과 Nelson 책위주로 정리하였고 논문도 따로 리뷰 했습니다.

목차

1. introduction
2. etiology
3. symptom
4. diagnosis
5. treatment
6. long-term follow up

본문내용

Introduction
Kawasaki disease(KD) was formerly known as mucocutaneous lymph node syndrome and infantile polyarteritis nodosa
Acute febrile illness of childhood
Highest incidence in Asian background
치료하지 않으면 동맥류를 포함한 관상동맥 합병증이 약 20%에서 발생

Etiology
Cause of KD is unknown, but certain epidemiologic and clinical features support infectious origin.
특정 연령층에 이환되고 지역적 및 계절적인 호발현상이 있다.
Infrequent occurrence of the illness in infants younger than 3 mo, likely the result of maternal antibodies, and absence of cases in adults
Genetic role in the pathogenesis of KD seems likely, as evidenced by higher risk in Asian children regardless of country of residenced

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Therapeutic management
The anticoagulation treatment in KD is used in level IV and V. It includes warfarin and LMWH.
In cases of treatment failure, then retreatment with IVIG is recommended or additional therapy with iv pulse of steroids (prednisolone or methylprednisolone).
Abciximab is a glycoprotein IIa/IIb inhibitor that is proposed in acute thrombotic events in KD, because of its ability for thrombi resolution, restoration of the arterial lumen in aneurysmatic dilatations.

Take home messages
Kawasaki disease (KD) is a vasculitis and a self-limited disease that affects mainly children.
The current pathogenetic hypothesis postulates activation of immune system.
There must be a genetic predisposition for some patients to develop the disease.
Management of KD is essential and patients should be treated properly by the tenth day of the disease.
The therapeutic management can be classified in this of the acute stage and the later on therapy.

참고자료

· 안효섭 편, 홍창의 소아과학 제10판(보정판), 서울: 대한교과서, 2010.
· Robert M. Kliegman, Nelson Textbook of Pediatrics, 19/e, W.B. Saunders, 2011, ISBN 1416040048
· Alexoudi I, Kanakis M/Kawasaki disease: current aspects on aetiopathogenesis and therapeutic management/Autoimmun Rev. 2011 Jul;10(9):544-7